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systemic necrotizing vasculitis

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https://www.readbyqxmd.com/read/29392482/ixazomib-induced-cutaneous-necrotizing-vasculitis
#1
A Alloo, H Khosravi, S R Granter, S M Jadeja, P G Richardson, J J Castillo, N R LeBoeuf
Ixazomib is a second-generation proteasome inhibitor that has been approved in the combination treatment of multiple myeloma and is currently under clinical investigation for the management of Waldenstrom's macroglobulinemia. While cutaneous adverse events secondary to proteasome inhibitors have been reported, the side effect profile of ixazomib remains to be documented. We report two patients, one with multiple myeloma and one with Waldenstrom's macroglobulinemia, who developed cutaneous necrotizing vasculitis after the initiation of ixazomib...
February 1, 2018: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
https://www.readbyqxmd.com/read/29368866/cutaneous-and-systemic-vasculitides-in-dermatology-a-histological-perspective
#2
Silvia Alberti-Violetti, Emilio Berti, Angelo V Marzano
Cutaneous vasculitides encompass a wide and heterogeneous group of diseases affecting skin blood vessels that are clinically characterized by polymorphic skin lesions, including in particular palpable purpura as well as urticarial and necrotic-ulcerative lesions, with possible, albeit rare, extracutaneous involvement. Cutaneous leukocytoclastic angiitis and urticarial vasculitis, which are the two prototypic and most common variants of this group, are usually idiopathic but may also be induced by different triggers, notably drugs and infections, or may manifest in association with systemic disorders, particularly lupus erythematosus...
January 24, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
https://www.readbyqxmd.com/read/29302827/colonic-perforation-due-to-severe-cytomegalovirus-disease-in-granulomatosis-with-polyangiitis-after-immunosuppression
#3
REVIEW
Alessandra Soriano, Nazareno Smerieri, Stefano Bonilauri, Loredana De Marco, Alberto Cavazza, Carlo Salvarani
Granulomatosis with polyangiitis (GPA) is a small-vessel necrotizing granulomatous vasculitis typically involving upper airways, lungs, and kidneys, which may lead to end-organ damage and life-threatening complications. Major infections during GPA course represent a considerable concern in the management of the disease. Cytomegalovirus (CMV) infection and disease are rare but significant complications in the course of GPA being associated with high morbidity and mortality rates. Colonic perforation due to CMV colitis is exceedingly rare and has so far almost exclusively been documented in HIV, renal transplant, and systemic lupus erythematosus patients...
January 4, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29282399/chronic-retinal-necrosis-severely-complicated-by-neovascular-glaucoma-a-case-report
#4
Takanori Matsuoka, Kazunobu Asao, Noriyasu Hashida, Kohji Nishida
Background: Chronic retinal necrosis (CRN) is a rare chronic granular necrotizing retinitis that was first described in 2013. CRN is characterized by intraocular inflammation accompanied by occlusive vasculitis, granular retinitis, and slowly progressing necrosis around the retina in a host with partial immune dysfunction. Cytomegalovirus (CMV) is reported to be a causative agent. There are several ocular complications such as retinal detachment and neovascular glaucoma; however, there has been no description of a clinical manifestation of neovascular glaucoma in CRN...
September 2017: Case Reports in Ophthalmology
https://www.readbyqxmd.com/read/29260748/-necrotizing-sarcoid-granulomatosis-with-clinical-presentations-of-recurrent-acute-abdomen-case-report-and-literature-review
#5
V I Vasilyev, S G Palshina, B D Chaltsev, S G Radenska-Lopovok, T N Safonova
The authors have described the world's first case of necrotizing sarcoid granulomatosis (NSG) in a 22-year-old woman with the clinical presentations of acute abdomen, which are associated with abdominal lymph nodal infiltration and necrosis, obvious constitutional disturbances (fever, nocturnal sweats, and significant weight loss), high inflammatory activity (anemia, leukocytosis, high erythrocyte sedimentation rates and C-reactive protein levels), the gradual appearance of splenic and hepatic necrotic foci, and infiltration into the lung and lacrimal glands with the development of unilateral uveitis...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/29260039/intracerebral-hemorrhage-after-iv-tpa-for-stroke-as-early-symptom-of-anca-associated-vasculitis
#6
Neda Zarghami Esfahani, Daniel M Anderson, Connie Pieper, Harold P Adams
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare diseases characterized by a necrotizing small-vessel vasculitis and circulating ANCA that comprise granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Acute ischemic stroke (AIS) can be a manifestation of central nervous system (CNS) involvement in these diseases. Furthermore, intracerebral hemorrhage (ICH) is a potential complication of these necrotizing vasculitides...
December 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29239918/anti-neutrophil-cytoplasmic-antibody-negative-central-nervous-system-granulomatosis-with-polyangiitis-and-its-clinical-characteristics
#7
Zhihua Chen, Yifeng Miao, Hui Wu, Ran Wang, Zhiyi Zhou, Shilei Zhang, Longtian Chen, Yongming Qiu
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous vasculitis occasionally affecting central nervous system (CNS), and GPA patients with initial CNS symptoms are even rarer, whose diagnosis is further confused by an absence of positive antineutrophil cytoplasmic antibody. The authors described the characteristics of antineutrophil cytoplasmic antibody -negative GPA with CNS onset in a patient and discussed on its management, which may contribute to future diagnosis and treatment of patients with similar conditions...
December 12, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29209584/sudden-death-from-spontaneous-coronary-artery-dissection-due-to-polyarteritis-nodosa
#8
Cyrus M Munguti, Paul M Ndunda, Tabitha M Muutu
Spontaneous coronary artery dissection (SCAD) is an emerging and rare cause of acute coronary syndrome and sudden cardiac death. While it was previously reported among young females with fibromuscular dysplasia, new literature indicates that this condition could occur in older populations. Polyarteritis nodosa (PAN) causes systemic necrotizing vasculitis which typically affects small to medium-sized muscular arteries and could affect the coronary arteries. A few case reports of PAN causing acute coronary artery disease have been reported in the literature...
October 2, 2017: Curēus
https://www.readbyqxmd.com/read/29189887/eosinophilic-granulomatosis-with-polyangiitis-without-respiratory-symptoms-or-asthma-in-an-adolescent-case-report-and-literature-review
#9
REVIEW
Gülçin Otar Yener, Zahide Ekici Tekin, Neşe Çallı Demirkan, Selçuk Yüksel
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain...
November 30, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29139157/seronegative-granulomatosis-with-polyangiitis-presenting-with-multiple-cranial-nerve-palsies
#10
Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung-Hye Park
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms...
November 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29127573/eosinophilic-granulomatosis-with-polyangiitis-complicated-by-subarachnoid-hemorrhage-and-coronary-vasculitis-a-case-report-and-review-of-the-literature
#11
REVIEW
Shogo Matsuda, Shuzo Yoshida, Youhei Fujiki, Hidetoshi Satomi, Tohru Takeuchi, Yoshinobu Hirose, Shigeki Makino, Shigeki Arawaka
Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by necrotizing vasculitis of small-sized vessels with extravascular granulomas and eosinophilic infiltration. The case of a 48-year-old Japanese woman with EGPA, who presented concurrently with subarachnoid hemorrhage (SAH) and coronary vasculitis, is reported. She initially presented with bronchial asthma, and then 8 months later she developed various symptoms caused by systemic eosinophilic vasculitis and was admitted to our hospital. Three days after admission, she started oral corticosteroid therapy, and her 2009 Five-Factor Score (FFS) was 0...
November 10, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29117463/vasculitis-and-iga-monoclonal-gammopathy-of-cutaneous-significance
#12
L Rousset, F Cordoliani, M Battistella, M Jachiet, E Pillebout, M Rybojad, M Bagot, E Oksenhendler, J-D Bouaziz
IgA vasculitis (Henoch-Schonlein purpura) is a small vessel systemic vasculitis involving IgA immunoglobulin polyclonal activation(1) . Monoclonal gammopathy of cutaneous significance is a subgroup of monoclonal gammopathy with skin disease, without myeloma or lymphoproliferative disorder(2) . We report 3 patients with vasculitis and IgA monoclonal gammopathy of cutaneous significance. Three men, 38, 37 and 50 years old, had a vascular purpura for more than 10 years with partial remission and frequent necrotic flares (Figure 1)...
November 8, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/29044308/heart-conduction-system-defects-and-sustained-ventricular-tachycardia-complications-in-a-patient-with-granulomatosis-with-polyangiitis-a-case-report-and-literature-review
#13
Laryssa Passos Sarmento Santos, Victor Guerreiro Bomfim, Camila Fagundes Bezerra, Natália Vieira Costa, Rafael Barreto Paes de Carvalho, Ricardo Sobral de Carvalho, Rogério da Hora Passos, Olivia Carla Bomfim Boaventura, André Luiz Nunes Gobatto
Granulomatosis with polyangiitis is a rare systemic inflammatory disorder characterized by vasculitis of the small arteries, the arterioles and the capillaries together with necrotizing granulomatous lesions. This case reports on a young female patient, previously diagnosed with granulomatosis with polyangiitis, who was admitted to the intensive care unit with seizures and hemodynamic instability due to a complete atrioventricular heart block. The event was associated with multiple episodes of sustained ventricular tachycardia without any structural heart changes or electrolyte disturbances...
July 2017: Revista Brasileira de Terapia Intensiva
https://www.readbyqxmd.com/read/29032100/-severity-of-illness-index-in-autoimmune-diseases-have-there-any-usefulness-in-medical-practice
#14
A Saunier, M-A Vandenhende, P Morlat, F Bonnet
INTRODUCTION: Assessing disease activity in patients suffering from autoimmune diseases is complex. Symptoms are multiple, often subjective and there are no reliable biomarkers. Many activity scores have been implemented to compare treatment efficacy in clinical trials. Their use in clinical practice is largely unknown. We performed a practical survey to analyze the use of activity scores in clinical practice to consider treatment response and to assess the determinants of their use. METHODS: A sample of French internists answered a questionnaire about activity scores of systemic lupus erythematosus, Sjögren's syndrome, autoimmune myositis and necrotizing vasculitis of small vessels...
November 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28992397/prognostic-significance-of-cavitary-lung-nodules-in-granulomatosis-with-polyangiitis-a-clinical-and-imaging-study-of-225-patients
#15
Bailey A Russell, Sindu Mohan, Rachandeep Chahal, Simon Carette, Christian Pagnoux
BACKGROUND: Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis with pulmonary nodules as a common manifestation. Our study examined whether pulmonary nodules, and nodule type (solid versus cavitary), are associated with different disease manifestations and outcomes. METHODS: Demographic, clinical, biological, radiological data at diagnosis, during follow-up, and treatments of GPA patients followed at the Mount Sinai Hospital (Canada) vasculitis clinic were analyzed...
October 9, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28941467/an-atypical-presentation-of-cardiac-tamponade-and-periorbital-swelling-in-a-patient-with-eosinophilic-granulomatosis-with-polyangiitis-a-case-report
#16
Alexandra C Keefe, Joseph C Hymas, Lyska L Emerson, John J Ryan
BACKGROUND: Eosinophilic granulomatosis with polyangiitis is a rare, necrotizing systemic vasculitis associated with asthma and hypereosinophilia. Its cause and pathophysiology are still being elucidated. CASE PRESENTATION: We report a case of eosinophilic granulomatosis with polyangiitis in a 50-year-old Caucasian woman who presented with chest pain, dyspnea at rest, fever, and periorbital swelling. She was found to have significant hypereosinophilia and cardiac tamponade physiology...
September 24, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28690751/-ocular-beh%C3%A3-et
#17
Ahmed Alami, Mohamed Kriet, Karim Reda, Abdelkader Laktaoui, Abdelbaare Oubaaz
Our study aimed to determine the clinical, therapeutic and prognostic features of ocular involvement in patients with Behçet's disease treated in our Department of ophthalmology. We conducted a retrospective data collection from medical records of 20 patients treated at the military hospital in Laayoune. All patients underwent complete ophthalmological examination and fluorescein angiography if necessary. OCT exam was performed in two patients. Ten patients had anterior uveitis, complicated in one case by ocular hypertonia; two patients had intermediate uveitis; eight patients had posterior segment involvement complicated in one case by intravitreal hemorrhage...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28678392/adding-azathioprine-to-remission-induction-glucocorticoids-for-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-microscopic-polyangiitis-or-polyarteritis-nodosa-without-poor-prognosis-factors-a-randomized-controlled-trial
#18
RANDOMIZED CONTROLLED TRIAL
Xavier Puéchal, Christian Pagnoux, Gabriel Baron, Thomas Quémeneur, Antoine Néel, Christian Agard, François Lifermann, Eric Liozon, Marc Ruivard, Pascal Godmer, Nicolas Limal, Arsène Mékinian, Thomas Papo, Anne-Marie Ruppert, Anne Bourgarit, Boris Bienvenu, Loïck Geffray, Jean-Luc Saraux, Elisabeth Diot, Bruno Crestani, Xavier Delbrel, Laurent Sailler, Pascal Cohen, Véronique Le Guern, Benjamin Terrier, Matthieu Groh, Claire Le Jeunne, Luc Mouthon, Philippe Ravaud, Loïc Guillevin
OBJECTIVE: In most patients with nonsevere systemic necrotizing vasculitides (SNVs), remission is achieved with glucocorticoids alone, but one-third experience a relapse within 2 years. This study was undertaken to determine whether the addition of azathioprine (AZA) to glucocorticoids could achieve a higher sustained remission rate of newly diagnosed nonsevere eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), microscopic polyangiitis (MPA), or polyarteritis nodosa (PAN)...
November 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28626961/comparing-polyarteritis-nodosa-in-children-and-adults-a-single-center-study
#19
Abdulsamet Erden, Ezgi D Batu, Hafize E Sönmez, Alper Sarı, Berkan Armagan, Zehra S Arıcı, Emre Bilgin, Umut Kalyoncu, Ömer Karadağ, Yelda Bilginer, Ali Ihsan Ertenli, Seza Özen
OBJECTIVE: Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium/small arteries. We aimed to examine the characteristics of adult- and childhood-onset PAN. METHODS: Fifteen pediatric (˂ 18 years) and 22 adult PAN patients who fulfilled the Ankara 2008 and American College of Rheumatology 1990 criteria, respectively, were included in the study. RESULTS: Five children had cutaneous and all the rest of the patients had systemic PAN...
August 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28623488/vasculitis-for-the-internist-focus-on-anca-associated-vasculitis
#20
REVIEW
Benjamin Chaigne, Loïc Guillevin
Vasculitides comprise several diseases affecting vessels of different sizes, mainly arteries, but also capillaries and veins. Antineutrophil cytoplasm antibody-associated vasculitides (AAVs) belong to the systemic necrotizing small-vessel vasculitis group that comprises granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Internists often see AAV patients, and although their outcomes are usually good, the disease can be responsible for morbidity and mortality...
August 2017: Internal and Emergency Medicine
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